Anesthetic management of a patient with Hallervorden-Spatz disease

Abstract

Hallervorden-Spatz disease (HSD) is a rare autosomal recessive disorder of the basal ganglia which follows a slowly progressive course from its onset in late childhood to death about ten years later. Dementia and dystonia with torticollis, scoliosis, and oromandibular rigidity are commonly present. There is no specific laboratory test diagnostic for this condition and no effective treatment is known. The diagnosis of HSD can only be made at autopsy unless a similarly affected sibling has died and demonstrated iron pigmentation in the globus pallidus and substantia nigra and widely disseminated focal axonal swelling.